Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly

A. Ameri; V. F. Fairbanks; G. A. Yanik; F. Mahdi; S. N. Thibodeau; D. J. McCormick; L. A. Boxer; K. T. McDonagh

doi:10.1182/blood.v94.5.1825

Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly

A. Ameri
, V. F. Fairbanks
, G. A. Yanik
, F. Mahdi
, S. N. Thibodeau
, D. J. McCormick
, L. A. Boxer
, K. T. McDonagh

Research output: Contribution to journal › Article › peer-review

8 Scopus citations

Abstract

We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the α(M)-globin chains that are more electropositive than normal α-globin chains.

Original language	English (US)
Pages (from-to)	1825-1826
Number of pages	2
Journal	Blood
Volume	94
Issue number	5
DOIs	https://doi.org/10.1182/blood.v94.5.1825
State	Published - Sep 1 1999
Externally published	Yes

ASJC Scopus subject areas

Biochemistry
Immunology
Hematology
Cell Biology

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Ameri, A., Fairbanks, V. F., Yanik, G. A., Mahdi, F., Thibodeau, S. N., McCormick, D. J., Boxer, L. A., & McDonagh, K. T. (1999). Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly. Blood, 94(5), 1825-1826. https://doi.org/10.1182/blood.v94.5.1825

Ameri, A, Fairbanks, VF, Yanik, GA, Mahdi, F, Thibodeau, SN, McCormick, DJ, Boxer, LA & McDonagh, KT 1999, 'Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly', Blood, vol. 94, no. 5, pp. 1825-1826. https://doi.org/10.1182/blood.v94.5.1825

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abstract = "We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the α(M)-globin chains that are more electropositive than normal α-globin chains.",

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AU - Ameri, A.

AU - Fairbanks, V. F.

AU - Yanik, G. A.

AU - Mahdi, F.

AU - Thibodeau, S. N.

AU - McCormick, D. J.

AU - Boxer, L. A.

AU - McDonagh, K. T.

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AB - We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the α(M)-globin chains that are more electropositive than normal α-globin chains.

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Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly

Abstract

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